Cushing's syndrome due to unilateral nodular adrenal hyperplasia: a new pathophysiological entity?

1980 ◽  
Vol 93 (4) ◽  
pp. 495-504 ◽  
Author(s):  
Robert G. Josse ◽  
Robert Bear ◽  
Kalman Kovacs ◽  
H. Patrick Higgins
Keyword(s):  
Adrenal Gland ◽  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Electron Microscopic Examination ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Adrenal Hyperplasia ◽  
Electron Microscopic ◽  
High Dose Dexamethasone ◽  
Steroid Excretion

Abstract. A case is presented of a 43-year-old Caucasian female with Cushing's syndrome, malignant hypertension and renal insufficiency. Plasma cortisol values were elevated (19.5 μg/100 ml at 0800 h and 18.5 μg/100 ml at 2200 h) with loss of diurnal variation and failure of suppression with low and high dose dexamethasone. 17-ketogenic steroid excretion remained unchanged after metyrapone administration. Adrenal vein plasma cortisol values showed prominent left-sided gradient. Gross, histologic and electron-microscopic examination of the left adrenal gland revealed changes of nodular adrenal hyperplasia. The presence of a suppressed right adrenal gland was confirmed post-operatively by a five day ACTH infusion. This case may represent a previously unreported variant of Cushing's syndrome due to unilateral nodular adrenal hyperplasia.

ORAL AND INTRAVENOUS SUPPRESSION TESTS IN THE DIAGNOSIS OF CUSHING'S SYNDROME

1965 ◽  
Vol 33 (3) ◽  
pp. 515-524 ◽  
Author(s):  
V. H. T. JAMES ◽  
J. LANDON ◽  
V. WYNN
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Early Period ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Adrenal Tumour ◽  
Bilateral Adrenal Hyperplasia ◽  
Steroid Excretion ◽  
Synthetic Glucocorticoids ◽  
Urinary Steroid

SUMMARY Adrenocortical suppression tests, based on the fall in urinary 17-hydroxy-corticosteroid excretion during the oral administration of dexamethasone, were found to be of value in the diagnosis of Cushing's syndrome, but less useful in differentiating bilateral adrenal hyperplasia from adrenal tumour. Such tests have the disadvantage of requiring accurate urine collections and of taking several days to perform. A test is described, based on the decrease in plasma cortisol concentration during i.v. infusion of dexamethasone at a rate of 1 mg./hr. The results obtained in 12 patients with Cushing's syndrome and bilateral adrenal hyperplasia differed from those found in control subjects in that there was a delay between the start of the infusion and the fall of plasma cortisol, and the rate of fall was less rapid. The values found after 180 min., expressed either as μg./100 ml. or as a percentage of the resting level, differed significantly (P < 0·001) in the two groups. The test proved valuable as an aid to the diagnosis of Cushing's syndrome, was easy to perform, and could be completed in 3 hr. In some patients with Cushing's syndrome, the administration of synthetic glucocorticoids appeared to result in an increased urinary steroid excretion. A transient increase in plasma cortisol levels was also observed in some of these patients during the early period of dexamethasone infusion. It is thought that this finding reflects an alteration in steroid metabolism induced by dexamethasone and fluorocortisol.

STEROID EXCRETION AND PLASMA CORTISOL IN 41 CASES OF CUSHING'S SYNDROME

1966 ◽  
Vol 51 (4) ◽  
pp. 511-525 ◽  
Author(s):  
Ingrid Ernest
Keyword(s):  
Cushing’S Syndrome ◽  
Plasma Cortisol ◽  
Cushing's Syndrome ◽  
Similar Data ◽  
Control Material ◽  
Present Material ◽  
Control Range ◽  
The Mean ◽  
Obesity Hypertension ◽  
Steroid Excretion

ABSTRACT Urinary excretion of 17-hydroxycorticosteroids (17-OHCS) and of Porter-Silber chromogens as well as plasma cortisol at different times of the day were measured repeatedly in 41 cases of Cushing's syndrome and the results were compared with similar data from 55 non Cushing patients suffering from obesity, hypertension and other symptoms often associated with Cushing's syndrome. The Cushing patients showed a marked variation in steroid output and in many cases the excretion was within the control range during one or several days. In only four of all patients, however, did the mean excretion of 17-OHCS overlap with that found in the control material. There was a marked overlapping between the Cushing and the non Cushing patients with respect to plasma cortisol. At midnight, however, most Cushing patients (34/36) at repeated determinations showed one or several figures higher than those of the control material. In the present material there was a marked overlapping between the Cushing (15 cases) and the non Cushing patients (14 cases) with respect to the increase in steroid excretion after intravenously administered corticotrophin (ACTH). In 14 cases of Cushing's syndrome due to hyperplasia the administration of metapyron caused an increase in steroid excretion. One such case failed to react to metapyron as did 3 cases of cortisol producing tumours.

scholarly journals A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

2019 ◽  
Vol 6 (3) ◽  
pp. 959
Author(s):  
Siddharth Pugalendhi ◽  
Tarun Kumar Dutta ◽  
Dhivya . ◽  
Kiran Yadav
Keyword(s):  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Somatostatin Analogues ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Ectopic Acth ◽  
High Dose Dexamethasone ◽  
Ectopic Acth Production ◽  
Duodenal Carcinoid

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years.  Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

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